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目的探讨IVIG无反应型川崎病(KD)的临床特点。方法分析总结2009年9月-2011年6月在本院风湿免疫科收治的KD患儿病例资料。IVIG无反应型定义为首次IVIG治疗48 h后体温仍超过38.5℃。初治有效者为敏感组,无效者为无反应组,对2组患儿临床表现及实验室数据进行统计分析。结果共378例患儿符合KD的诊断标准,并接受IVIG治疗。其中敏感者354例;无反应者24例,无反应发生率为6.35%;无反应组与敏感组相比,多型性红斑及颈部淋巴结大触痛明显,全血WBC、LDH显著升高,Hb及清蛋白(ALB)显著降低(Pa<0.05),无反应组冠状动脉病变的发生率较敏感组明显升高(P<0.05);无反应组再次治疗的选择中,7例接受追加IVIG 2 g·kg-1治疗,17例接受激素治疗,均热退。接受追加IVIG和接受激素治疗的2个IVIG无反应再次治疗组间冠状动脉病变发生率比较差异无统计学意义。结论 IVIG无反应型KD的特点为多型性红斑及颈部淋巴结大触痛明显,WBC、LDH较高,Hb及ALB较低,冠状动脉病变发生率高。
Objective To investigate the clinical features of non-responsive Kawasaki disease (KD) in IVIG. Methods The data of KD children admitted to the Department of Rheumatology from September 2009 to June 2011 were analyzed and summarized. IVIV nonresponsiveness was defined as the body temperature was still above 38.5 ° C 48 hours after the first IVIG treatment. The newly diagnosed patients were sensitive group, and those who were ineffective were nonresponders. The clinical manifestations and laboratory data of two groups were statistically analyzed. Results A total of 378 children met the KD diagnostic criteria and received IVIG. Among them, 354 cases were sensitive and 24 cases were non-responders, the rate of non-reaction was 6.35%. Compared with the sensitive group, polymorphous erythema and cervical lymph node large tenderness were obvious, WBC and LDH in whole blood were significantly increased (P <0.05). The incidence of coronary artery lesion in non-reaction group was significantly higher than that in the sensitive group (P <0.05). Among the non-response group, 7 patients received additional IVIG 2 g · kg-1 treatment, 17 patients received hormone therapy, are hot retreat. There was no significant difference in the incidence of coronary artery lesions between the two IVIG non-responders receiving additional IVIG and hormone therapy. Conclusions IVIG nonresponsive KD is characterized by large erythema multiforme and large cervical lymph node pain, high WBC and LDH, low Hb and ALB, and high incidence of coronary artery lesions.